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1.
G Ital Nefrol ; 36(2)2019 Apr.
Artigo em Italiano | MEDLINE | ID: mdl-30983177

RESUMO

Thrombotic microangiopathies (TMA) are a group of diseases that can complicate pregnancy and threaten the lives of both the mother and the fetus. Several conditions can lead to TMA, including thrombotic thrombocytopenic purpura (TTP), HELLP syndrome and hemolytic uremic syndrome (HUS). We describe the case of a 39-year-old woman who presented a HELLP syndrome in the immediate postpartum period. The patient had acute kidney injury (AKI), increased LDH, unmeasurable haptoglobin levels and hypocomplementemia. Her ADAMTS13 value was normal, thus ruling out TTP. Shiga toxin tests were negative, so HUS associated with E. coli was also ruled out. HELLP syndrome and atypical hemolytic-uremic syndrome (aHUS) remained the most probable diagnosis. In the days following childbirth, the patient's transaminase and bilirubin levels normalized while the anemia persisted, as did the AKI, resulting in the institution of dialysis treatment. A diagnosis of aHUS was made and therapy with eculizumab was started. The patient's blood counts progressively improved, urine output was restored, her indices of renal function also concomitantly improved and dialysis was interrupted. A rash appeared after the third administration of eculizumab and the treatment was suspended. The patient is currently being followed up and has not relapsed. At thirteen months after delivery her renal function is normal as are her platelet counts, LDH, haptoglobin levels and proteinuria. Tests for mutations in the genes that regulate complement activity were negative. We believe that childbirth triggered the HELLP syndrome, which in turn brought about and sustained the HUS. In fact, the patient's liver function improved right after delivery, while her kidney injury and hemolysis persisted, and she also had an excellent response to eculizumab. To our knowledge, no other cases of HELLP syndrome associated with haemolytic uremic syndrome during pregnancy have been reported in literature, nor have cases in which treatment with eculizumab was limited to only three administrations.


Assuntos
Injúria Renal Aguda/complicações , Síndrome Hemolítico-Urêmica Atípica/etiologia , Síndrome HELLP/etiologia , Adulto , Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome Hemolítico-Urêmica Atípica/terapia , Inativadores do Complemento/uso terapêutico , Feminino , Síndrome HELLP/terapia , Humanos , Doenças do Sistema Imunitário/complicações , Período Pós-Parto , Gravidez , Diálise Renal
2.
G Ital Nefrol ; 34(3): 44-53, 2017 Jun.
Artigo em Italiano | MEDLINE | ID: mdl-28700182

RESUMO

A pseudoaneurysm or false aneurysm of the brachial artery is an uncommon occurrence in patients receiving hemodialysis with arteriovenous fistula (AVF). We describe the case of a 76-year-old woman presenting with a large, tender, pulsatile mass in the right antecubital region 10 cm from the AVF. B-mode ultrasound examination revealed a saccular hematoma. Color doppler showed a recirculation movement of blood, creating a two colors image called "Korean flag". The patient was transferred to the surgical unit where she first underwent ultrasound-guided compression and then surgical repair of the pseudoaneurysm. Two weeks after surgery the AVF was used again as an access for hemodialysis. Differentiating between a false and a true aneurysm based on ultrasound is not always straightforward. Doppler ultrasound findings can be decisive for the early diagnosis of a pseudoaneurysm to ensure proper treatment planning given the dangerous complications of ruptures. Treatment options include: compression, percutaneous thrombin injection, endovascular exclusion with covered stents, aneurysmectomy and surgical repair.


Assuntos
Falso Aneurisma/etiologia , Derivação Arteriovenosa Cirúrgica/efeitos adversos , Artéria Braquial , Complicações Pós-Operatórias/etiologia , Diálise Renal , Idoso , Feminino , Humanos , Índice de Gravidade de Doença
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